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2.1
Dysgammaglobulinemia
Dysgammaglobulinemias

Presentation

Discussions on the immunoregulation and immunological tolerance by T cells and the growth control of mammalian cells are also presented in this volume. [books.google.com]

For the latter reason, the term immunoglobulin has been proposed to denote these proteins.2 At the present time three major classes of immunoglobulins are recognized, designated gamma2, gamma1A and gamma1M. [nejm.org]

The present report is primarily concerned with the roentgenologic appearance of these nodular defects in 10 patients. [pubs.rsna.org]

Entire Body System

  • Asymptomatic

    Excerpt To the Editor: I would like to disagree with a statement made by the authors of the paper entitled, "'Asymptomatic' Type I Dysgammaglobulinemia in Siblings" (Ann. Intern. Med. 68: 867, 1968). [acpjournals.org]

    Though affected persons may be asymptomatic, low levels of IgA will reduce the immune system's ability to combat infection where IgA is normally secreted, at mucosal surfaces. [ebi.ac.uk]

    Study of an 11-year-old girl with recurrent severe infections associated with dysgammaglobulinemia, lymphopenia and lymphocytotoxic antibody, resulting in loss of immunologic memory. ( 4170529 ) Kretschmer R....Rosen F.S. 1968 50 "Asymptomatic&quot [malacards.org]

    The immunodeficiency has been associated with some clinical disorders including recurrent infections, atopy, Bloom's syndrome, celiac disease, systemic lupus erythematosus and malignancy, but, surprisingly, SIgMD seems to also occur in asymptomatic individuals [howlingpixel.com]

    Asymptomatic patients with XLP should be periodically monitored for immunoglobulin levels, evidence for EBV exposure, and signs of development of other manifestations of disease. [atlasgeneticsoncology.org]

  • Epilepsy

    […] telangiectasia 0 *Dysgammaglobulinemia *Epilepsy *Intellectual Disability *Telangiectasis. [reference.md]

    Return to Article Details Association of severe myoclonic epilepsy of infancy (SMEI) with probable autoimmune lymphoproliferative syndrome-variant Download Download PDF [pediatrmedchir.org]

    […] leucoencephalopathy in a 58-year-old man with XMEN disease. 62 Dhalla F...Patel SY 25504528 2015 34 Dysgammaglobulinemia Associated With Glu349del, a Hypomorphic XIAP Mutation. 62 Nishida N...Kanegane H 26182687 2015 35 Association of severe myoclonic epilepsy [malacards.org]

    Immunoglobulin treatment in epilepsy, a review of the literature. Epilepsy Res 1994;19(3):181-90. [ Links ] 200. Baskin E, Dener S, Gültein A, Tanzer F, Sekreter E, Türkay S. Immune globulin treatment in intractable epilepsy of childooh. [scielo.org.mx]

  • Lymphadenopathy

    Interestingly enough, many conditions with lymphadenopathy and dysgammaglobulinemia share similar histologic features, such as infiltration with lymphocytes, immunoblasts, plasma cells, and histiocytes. [ncbi.nlm.nih.gov]

    We observed lymphadenopathy (LAD), cytopenias, liver disease, cavum septum pellucidum (CSP), and increased CD4-CD8-B220-TCRαβ+ T cells (αβDNTs), in addition to the previously described features of an inverted CD4/CD8 ratio, CD4+ T lymphocytopenia, increased [profiles.wustl.edu]

    M.E....Ikeda R.M. 1980 24 Inherited 7 S immunoglobulin deficiency in chickens: late onset dysgammaglobulinemia and evaluation of the lymphocyte proliferative responses and other immunologic functions. ( 6893299 ) Benedict A.A....Gershwin M.E. 1980 25 Lymphadenopathy [malacards.org]

    Abnormal enlargement of the spleen (splenomegaly) and the lymph nodes (lymphadenopathy) because of an increase in the number of white blood cells within lymph nodes (lymphoid hyperplasia) are also common. [rarediseases.org]

  • Recurrent Pleural Effusion

    A case report and family study. ( 163713 ) Bader P.I....Nance W.E. 1975 31 Correction of neutropenia associated with dysgammaglobulinemia. ( 4137595 ) Rieger C.H....Rothberg R.M. 1974 32 Recurrent pleural effusion associated with dysgammaglobulinemia. [malacards.org]

  • Short Stature

    Overview A very rare syndrome characterized mainly by low birth weight, short stature and a immune system abnormality. [checkorphan.org]

Respiratoric

  • Pleural Effusion

    A 45-year-old man presented with recurrent bilateral pleural effusions of undetermined etiology. This was associated with absence of IgM and a decreased IgA fraction. Possible associations are discussed. [journal.chestnet.org]

    A case report and family study. ( 163713 ) Bader P.I....Nance W.E. 1975 31 Correction of neutropenia associated with dysgammaglobulinemia. ( 4137595 ) Rieger C.H....Rothberg R.M. 1974 32 Recurrent pleural effusion associated with dysgammaglobulinemia. [malacards.org]

    Chest radiography revealed severe pneumonia with bilateral pleural effusion. [mdpi.com]

Neurologic

  • Amnesia

    A case report. ( 5642298 ) Grise J.W. 1968 49 Immunologic amnesia. [malacards.org]

Workup

A: I believe that your workup is sufficient. Copied below, for your interest, is a posting from our “Ask the Expert” website in response to a similar inquiry. This will give you references to pursue in regards to your question. [aaaai.org]

Clinical Testing and Workup Blood tests will be ordered to determine the status of immunoglobulins in the blood, including normal or high levels of IgM and low levels of other immunoglobulin classes. [rarediseases.org]

He was further investigated at this point when a routine immunological workup showed an elevated IgM level with low IgG and IgA levels, prompting the need to consider hyper IgM syndrome (Table 1). [mdpi.com]

Serum

  • Lymphopenia

    She manifested recurrent, profound lymphopenia in association with infection. [nature.com]

    Study of an 11-year-old girl with recurrent severe infections associated with dysgammaglobulinemia, lymphopenia and lymphocytotoxic antibody, resulting in loss of immunologic memory. ( 4170529 ) Kretschmer R....Rosen F.S. 1968 50 "Asymptomatic&quot [malacards.org]

    […] to be a more general theme in the immune system and is to some degree reminiscent of the reconstitution phase after T-cell lymphopenia. [bloodjournal.org]

    In summary, if your patient does not have a significant lymphopenia (less than 750/uL), I would administer the zoster vaccine. I would document a discussion of the issues since this is a live virus vaccine. [aaaai.org]

    Congenital chylothorax: lymphopenia and high risk of neonatal infections. Acta Paediatr 2004;93:220-224. [ Links ] 92. Ramaiah RN, Cale CM, Hartley JC, Brown KL, Hoskote AU. [scielo.org.mx]

  • Hyperglobulinemia

    The occurrence of serological “antibody” reagins or similar γ-globulins in conditions with monoclonal hyperglobulinemia, such as myeloma, macroglobulinemia, etc.. [amjmed.com]

Microbiology

  • Streptococcus Pneumoniae

    Selective immunoglobulin M deficiency in an adult with Streptococcus pneumoniae sepsis and invasive aspergillosis. J Investig Allergol Clin Immunol. 2008. 18(3):214-8. [Medline]. [emedicine.medscape.com]

Pleura

  • Pleural Effusion

    A 45-year-old man presented with recurrent bilateral pleural effusions of undetermined etiology. This was associated with absence of IgM and a decreased IgA fraction. Possible associations are discussed. [journal.chestnet.org]

    A case report and family study. ( 163713 ) Bader P.I....Nance W.E. 1975 31 Correction of neutropenia associated with dysgammaglobulinemia. ( 4137595 ) Rieger C.H....Rothberg R.M. 1974 32 Recurrent pleural effusion associated with dysgammaglobulinemia. [malacards.org]

    Chest radiography revealed severe pneumonia with bilateral pleural effusion. [mdpi.com]

Treatment

Spiramycin was added toward the end of the treatment period. This patient failed to show clinical response although there was a decrease in the stool oocyst count from the value just prior to therapy. [ncbi.nlm.nih.gov]

At the visit, write down the name of a new diagnosis, and any new medicines, treatments, or tests. Also, write down any new instructions your provider gives you. Know why a new medicine or treatment is prescribed, and how it will help you. [hopkinsmedicine.org]

The use of immunoglobulins in the treatment of human epilepsy. Neurol Sci 2002;23(1):33-37. [ Links ] 202. Reiner WO, Weemaes CM, van Engelen BG. Immunoglobulin treatment in human and experimental epilepsy. [scielo.org.mx]

Página 414 - Report of the National Cholesterol Education Program (NCEP) Expert Panel on detection, evaluation, and treatment of high blood cholesterol in adults (Adult Treatment Panel II). ‎ [books.google.es]

This service should be used for second opinions, additional medical reviews and consultations; not for primary diagnosis or treatment. [xmri.com]

Prognosis

Prognosis - Low birth weight dwarfism dysgammaglobulinemia Not supplied. Treatment - Low birth weight dwarfism dysgammaglobulinemia Not supplied. Resources - Low birth weight dwarfism dysgammaglobulinemia Not supplied. [checkorphan.org]

The prognosis has been poor, with little efficacy for corticosteroids, splenectomy, or thymectomy. [neutropenia.ca]

PMID: 3286728 Prognosis Tragiannidis A, Groll AH Paediatr Drugs 2021 Sep;23(5):445-455. Epub 2021 Jul 22 doi: 10.1007/s40272-021-00461-3. PMID: 34292515 Wall LA, Dimitriades VR, Sorensen RU Immunol Allergy Clin North Am 2015 Nov;35(4):659-70. [ncbi.nlm.nih.gov]

The prognosis is similarly poor for XLP2 without HCT, in which mortality is attributed to HLH (30%), colitis (23%), liver failure (8%), and infectious causes (8%) (Pachlopnik Schmid et al., 2011). [atlasgeneticsoncology.org]

Etiology

It is obvious that more knowledge of etiologic factors and pathogenetic mechanisms is necessary to classify, cure, and eventually prevent the diseases described in this paper. [ncbi.nlm.nih.gov]

A 45-year-old man presented with recurrent bilateral pleural effusions of undetermined etiology. This was associated with absence of IgM and a decreased IgA fraction. Possible associations are discussed. [journal.chestnet.org]

They are organised into groups, and further divided into clinical, etiological or histopathological sub-types. [orpha.net]

Viral culture or examintion of vesicle contents by indirect immunofluorescent technic can provide definitive diagnosis of the etiology of a zosteriform eruption. Clin Infect Dis. 2010 Jul 15;51(2):197-213. [aaaai.org]

Epidemiology

Epidemiology Frequency United States In a retrospective study of a large allergy practice (20,000 patients) database, Goldstein et al reported prevalences of SIgM deficiency of 0.26% among adults and 0.03% among children. [3, 4] International SIgM deficiency [emedicine.medscape.com]

Epidemiology, outcome, targeted agents and immunotherapy in adolescent and young adult non-Hodgkin and Hodgkin lymphoma. Br J Haematol. 2019;185:1142–57. https://doi.org/10.1111/bjh.15789. https://doi.org/10.1111/bjh.15789 Dumaswala K, Mehta A. [ouci.dntb.gov.ua]

Pathophysiology

Although the book’s primary focus is on practical diagnosis and management, the pathophysiology of PID is also discussed. [books.google.com]

Together, these data provide new clinical and pathophysiological foundations with important ramifications for the diagnosis and treatment of XMEN disease. APA Author BIBTEX Harvard Standard RIS Vancouver [profiles.wustl.edu]

Myelokathexis Intramedullary destruction of neutrophils appears to be the pathophysiologic basis of myelokathexis. Patients demonstrate moderate neutropenia with morphologic abnormality of the neutrophilic nuclei. [neutropenia.ca]

This reflects major differences in the pathophysiology of latency and reactivation of these 2 alphaherpesviruses. [aaaai.org]

Pathophysiology The cause of SIgM deficiency is unknown. [emedicine.medscape.com]

Prevention

Rapidly find the answers you need with separate sections on diseases and disorders, differential diagnosis, clinical algorithms, laboratory results, and clinical preventive services, plus an at-a-glance format that uses cross-references, outlines, bullets [books.google.com]

It is obvious that more knowledge of etiologic factors and pathogenetic mechanisms is necessary to classify, cure, and eventually prevent the diseases described in this paper. [ncbi.nlm.nih.gov]

Treatment with tacrolimus was successful in preventing disease relapses. This case stresses the relevance of central nervous system anti-aquaporin-4 auto-immunity in a broader context of immune dysregulation and neuro-immunology. [guthyjacksonfoundation.org]

The Lipid Research Clinics Coronary Primary Prevention Trial Results, II: the relationship of reduction in incidence of coronary heart disease to cholesterol lowering. ‎ [books.google.es]

Prevention - Low birth weight dwarfism dysgammaglobulinemia Not supplied. Diagnosis - Low birth weight dwarfism dysgammaglobulinemia Not supplied. Prognosis - Low birth weight dwarfism dysgammaglobulinemia Not supplied. [checkorphan.org]

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2.1
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